Selection of antigen receptors in splenic marginal-zone lymphoma: further support from the analysis of the immunoglobulin light-chain gene repertoire

We recently demonstrated that >30% of the cases with splenic marginal-zone lymphoma (SMZL) express B-cell receptors (BcRs) that carry a single polymorphic variant of the IGHV1-2 gene (IGHV1-2*04).1 The IGHV1-2*04 BcRs in SMZL are characterized by long complementarity-determining region-3 (CDR3) and exhibit a low impact of somatic hypermutation (SHM), leading to limited changes, which cluster in certain positions of the VH domain. Altogether, these features argue for selection by antigen in the pathogenesis of SMZL and suggest heavy chain (HC) dominance in the clonogenic immunoglobulin (IG) receptors of SMZL.